Diagnostic criteria for cryopyrin-associated periodic syndrome (CAPS)

JB Kuemmerle-Deschner, S Ozen, PN Tyrrell… - Annals of the …, 2017 - ard.bmj.com
Cryopyrin-associated periodic syndrome (CAPS) is a rare, heterogeneous disease entity
associated with NLRP3 gene mutations and increased interleukin-1 (IL-1) secretion. Early …

Sex differences in pediatric rheumatology

M Cattalini, M Soliani, MC Caparello… - Clinical reviews in allergy & …, 2019 - Springer
Autoimmune diseases affect up to 10% of the world's population and, as a whole, they are
far more common in females, although differences exist according to the single disease and …

Behçet's disease in children, an overview

I Koné-Paut - Pediatric Rheumatology, 2016 - Springer
BD is a systemic inflammatory disease with a variable vasculitis. Paediatric onset is very rare
and carries a strong genetic component. Oral ulcers and fever of unknown origin are …

A20 haploinsufficiency (HA20): clinical phenotypes and disease course of patients with a newly recognised NF-kB-mediated autoinflammatory disease

FA Aeschlimann, ED Batu, SW Canna, E Go… - Annals of the …, 2018 - ard.bmj.com
Objectives The association between mutations in TNFAIP3, encoding the NF-kB regulatory
protein A20, and a new autoinflammatory disease has recently been recognised. This study …

Changes in the proportion of clinical clusters contribute to the phenotypic evolution of Behçet's disease in Japan

Y Soejima, Y Kirino, M Takeno, M Kurosawa… - Arthritis Research & …, 2021 - Springer
Abstract Background We hypothesized that Behçet's disease (BD) consists of several clinical
subtypes with different severity, resulting in heterogeneity of the disease. Here, we …

French recommendations for the management of Behçet's disease

I Kone-Paut, S Barete, B Bodaghi, K Deiva… - Orphanet journal of rare …, 2021 - Springer
Behçet's disease (BD) is a systemic variable vessel vasculitis that involves the skin, mucosa,
joints, eyes, arteries, veins, nervous system and gastrointestinal system, presenting with …

Pediatric Behçet's disease

M Yildiz, F Haslak, A Adrovic, S Sahin, O Koker… - Frontiers in …, 2021 - frontiersin.org
Behçet's Disease (BD) is a systemic vasculitis firstly described as a disorder causing
aphthous lesion in oral and genital mucosae and uveitis. The disease has an extremely …

Intestinal Behçet and Crohn's disease: two sides of the same coin

S Valenti, R Gallizzi, D De Vivo, C Romano - Pediatric Rheumatology, 2017 - Springer
Behçet's disease (BD) and Crohn's disease (CD) are chronic immune-mediated,
inflammatory disorders affecting many different systems (joints, skin, eyes, gastrointestinal …

Childhood vasculitis

A Schnabel, CM Hedrich - Frontiers in pediatrics, 2019 - frontiersin.org
The term vasculitis covers heterogeneous disorders that share the presence of inflammation
of blood vessel walls. Immune cell infiltrates can vary significantly and involve granulocytes …

Clinical characteristics and genetic analyses of 187 patients with undefined autoinflammatory diseases

NM Ter Haar, C Eijkelboom, L Cantarini… - Annals of the …, 2019 - ard.bmj.com
Objectives To describe the clinical characteristics, treatment response and genetic findings
in a large cohort of patients with undefined systemic autoinflammatory diseases (SAIDs) …